Functional C1-inhibitor diagnostics in hereditary angioedema: assay evaluation and recommendations.

@article{WagenaarBos2008FunctionalCD,
  title={Functional C1-inhibitor diagnostics in hereditary angioedema: assay evaluation and recommendations.},
  author={Ineke G. A. Wagenaar-Bos and Christian Drouet and Emel Ayg{\"o}ren-Pursun and Konrad Bork and Christoph Bucher and Anette Bygum and Henriette Farkas and G Fust and Hanna Gregorek and Cornelis Erik Hack and Alaco Hickey and Helen I. Joller-Jemelka and Maria Kapusta and Wolfhart Kreuz and Hilary Longhurst and Margarita L{\'o}pez-Trascasa and Kazimierz Madaliński and Jerzy Naskalski and Ed J. Nieuwenhuys and Denise Ponard and Lennart Truedsson and Lilian Varga and Erik Waage Nielsen and Eric Wagner and Lorenza Chiara Zingale and Marco Cicardi and S. Marieke van Ham},
  journal={Journal of immunological methods},
  year={2008},
  volume={338 1-2},
  pages={
          14-20
        }
}
Hereditary angioedema (HAE) is an autosomal dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. The most widespread underlying genetic deficiency is a heterozygous deficiency of the serine protease inhibitor C1 esterase inhibitor (C1-Inh). In addition to low C4 levels, the most important laboratory parameter for correct diagnosis of HAE or angioedema due to acquired C1-Inh deficiency is reduced C1-Inh function (fC1-Inh). No direct recommendations… CONTINUE READING
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Dutch patients and the patient association. Abstracts from C1 Inhibitor deficiency workshop

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1 Excerpt

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